Treatment of Familial Mediterranean Fever with Canakinumab in Patients Who Are Unresponsive to Colchicine

Overview

Journal Eur J Rheumatol

Publisher Aves

Specialty Rheumatology

Date 2019 Aug 1

PMID 31365342

Citations 11

Authors

Afig Berdeli

Ozgur Senol

Gamze Talay

Affiliations

Soon will be listed here.

Abstract

Objective: Familial Mediterranean fever (FMF) is the most common inherited monogenic autoinflammatory disease worldwide. It is caused by loss-of-function mutations in the MEFV gene, mostly affecting Eastern Mediterranean population. It is discussed if it should be considered an autosomal-dominant disease with variable penetrance, because heterozygosis mutations are associated with clinical autoinflammatory manifestations. Colchicine constitutes that the mainstay of FMF treatment should be preventing acute attacks and amyloidosis, and decreasing the chronic inflammation. In colchicine-resistant or intolerant patients, recent insights into the pathogenesis of FMF have made the anti-IL1 treatments important. We aimed to search for the retrospective results of canakinumab treatment in patients with FMF who are unresponsive to colchicine.

Methods: In this study, 22 (13 males and nine females) patients with FMF with colchicine resistance/intolerance, age ranging from 6 to 18 years, were included in Ege University Department of Pediatric Rheumatology. After clinical and genetic diagnosis, colchicine treatment with standard doses was started. After treatment with canakinumab, complete response to treatment was determined as no acute episodes and normal level of acute phase reactants.

Results: After canakinumab treatment, 22 patients with FMF who were colchicine-resistant were evaluated. After the treatment, no attack was observed in 19 patients, and the values of acute phase reactants were normal in 22 patients. In three patients, disease attack was observed 16 months after the first dose treatment. In all patients, the values of acute phase reactants were found at normal level during treatment. No drug-related side effects were observed in any patient.

Conclusion: Canakinumab is an effective and safe anti-IL1 agent to reduce attacks in patients with FMF with no response to colchicine and to reduce the level of high-level laboratory findings associated with FMF.

Citing Articles

A narrative review on the role of cytokines in the pathogenesis and treatment of familial Mediterranean fever: an emphasis on pediatric cases.

Chaaban A, Yassine H, Hammoud R, Kanaan R, Karam L, Ibrahim J Front Pediatr. 2024; 12:1421353.

PMID: 39132307 PMC: 11310175. DOI: 10.3389/fped.2024.1421353.

The effect of canakinumab treatment on growth parameters in children with familial Mediterranean fever.

Arslanoglu Aydin E, Baglan E, Kocamaz N, Bagrul I, Tuncez S, Ozdel S Clin Rheumatol. 2023; 43(1):387-392.

PMID: 37658934 DOI: 10.1007/s10067-023-06752-z.

Effect of interleukin-1 antagonist on growth of children with colchicine resistant or intolerant FMF.

Pinchevski-Kadir S, Gerstein M, Pleniceanu O, Yacobi Y, Vivante A, Granat O Pediatr Rheumatol Online J. 2023; 21(1):4.

PMID: 36624531 PMC: 9827626. DOI: 10.1186/s12969-022-00784-6.

Evaluation of periodontal status and cytokine response in children with familial Mediterranean fever or systemic juvenile idiopathic arthritis.

Acar B, Demir S, Ozsin-Ozler C, Tan C, Ozbek B, Yaz I Clin Oral Investig. 2022; 27(3):1159-1166.

PMID: 36197547 DOI: 10.1007/s00784-022-04730-4.

Real-Life Indications of Interleukin-1 Blocking Agents in Hereditary Recurrent Fevers: Data From the JIRcohort and a Literature Review.

Vinit C, Georgin-Lavialle S, Theodoropoulou A, Barbier C, Belot A, Mejbri M Front Immunol. 2021; 12:744780.

PMID: 34858402 PMC: 8632237. DOI: 10.3389/fimmu.2021.744780.

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